IgA肾病的实验室检查及其意义

1引言 IgA肾病是一免疫病理学诊断名称,指肾小球系膜区以IgA或IgA沉积为主的原发性肾小球疾病.IgA1肾病在亚太地区和西欧分别占原发性肾小球疾病的20%～40%和10%～30%,是常见的肾小球疾病之一.IgA肾病临床表现多样,从无症状血尿和(或)蛋白尿到急进性肾小球肾炎都可能出现,而以发作性肉眼血尿和镜下血尿最多见,约10%～20%表现为肾病综合征.在我国,IgA肾病也是最常见的原发性肾小球疾病,约占原发性肾小球疾病的40%,约有20%～40%的IgA肾病患者可在20～25年内发展为终末期肾病,占终末期肾病近1/3,已成为终末期肾病的主要病因之一.     

p21、p27和PCNA在IgA肾病系膜细胞中的表达水平研究

IgA肾病是最常见的原发性肾小球肾炎，是导致终末期肾功能衰竭最常见的原因之一，也是我国最常见的原发性肾小球疾病，发病率较高，占原发性肾小球疾病的30％～40％。IgA肾病肾小球系膜区有以IgA免疫复合物为主的颗粒样沉积，同时有系膜细胞的增生，基质增多，系膜区电子致密物沉积。通过长期观察发现约20％的IgA肾病患者经20年临床疾病演变后进展至终末期肾功能衰竭。现通过对肾脏不同病理损害程度系膜细胞中p21、p27、PCNA的表达水平，进一步研究抑制系膜细胞增生的调控因子。     

IgA肾病与基因多态性

IgA肾病(IgA nephropathy,IgAN)是指肾小球系膜区以IgA或IgA沉积为主的原发性肾小球疾病。IgA肾病是肾小球源性血尿最常见的病因,是引起终末期肾功能衰竭的重要原因之一。在中国占原发性肾炎患者的25%~50%。IgA肾病的发病机制目前尚未完全清楚,近十多年来,随着分子生物学研究手     

IgA肾病的治疗

IgA肾病是常见的肾脏疾病。亚州及澳大利亚是IgA肾病的高发区,最高可达原发性肾小球肾炎的40%。我国不完全统计表明,其发病率为20～30%,我院资料为29%。近年来大量研究证明,IgA肾病并非为良性疾病,20～30%患者缓慢进展到肾功能衰竭。一般来讲,出现临床症状后10年,13%已发生终末期肾功能衰竭,20年时为26%。中国人IgA肾病患者的特点之一是大量蛋白尿哦肾病综合征占比例较大,所以肾功能衰竭的发生率可能更高,进展速度也会更     

柴苓汤治疗原发性IgA肾病的效果评估

免疫球蛋白A（immunoglobulin A,IgA）肾病是常见的原发性肾小球疾病,15%~40%的IgA肾病患者在10~20年后进展为终末期肾病。     

浅析IgA肾病患者的病理与临床关系

IgA肾病(IgA nephropathy，IgAN)是世界范围内最常见的肾小球疾病．也是主要引起终末期肾病的疾病之一，在我国亦是最常见的原发性肾小球疾病之一，其发病率占原发性肾小球疾病的26％～34％。据报道，IgAN发病后约9％～50%患者于20年内可逐渐发展为终末期肾病(end-stage renal disease，ERSD)。     

霉酚酸酯治疗IgA肾病的系统评价

免疫球蛋白A(IgA)肾病是以IgA或IgA为主的免疫球蛋白在肾小球系膜弥漫沉积为特征的肾小球肾炎。原发性IgA肾病是我国最常见的肾小球疾病之一,是导致终末期肾功能衰竭的一个主要原因[1]。IgA肾病在临床上可表现为反复肉眼血尿、大量蛋白尿、急进性肾炎综合征等。IgA肾病的治疗应     

成人原发性IgA肾病伴IgM沉积的临床病理特点分析

正IgA肾病(IgAN)是最常见的原发性肾小球疾病,是导致成年人终末期肾病的主要病因,病理特点是肾小球系膜区和(或)毛细血管袢以多聚IgA1为主及补体成分的沉积,伴肾小球系膜细胞增殖及系膜区基质增多。IgAN患者30年内约50%进展为终末期肾病[1,2]。IgAN进展缓慢,许多因素与IgAN预后密切相关,如蛋白尿、高血压及肾功能损伤程度等,除此之外,研究发现免疫球蛋白IgG、补     

IgA肾病的临床特征分析

IgA肾病是原发性肾小球肾炎中最常见的一种独立性疾病,占原发性肾小球疾病的26％～34％,是导致终末期肾功能衰竭的主要原因之一。为了解IgA肾病的临床特征,以提高临床诊断、治疗水平及判断预后,作者对近年来收治的40例IgA肾病的临床、病理和免疫荧光资料进行分析,结果报道如下。     

IgA肾病中半乳糖缺乏的IgA1及其相关标志物的研究进展

正IgA肾病是最常见的肾小球疾病之一,患者临床主要表现为血尿、不同程度的蛋白尿和肾功能受损,病理表现则为肾小球系膜区以IgA1和补体C3为主的沉积或是伴有IgG、IgM的共沉积[1]。最近日本一项大规模研究报道显示,大约50%的IgA肾病患者在诊断后的30年内进展为终末期肾病[2]。可见,通过生物标志物评估患者的预后和进展变化对于临     

508例肾活检资料的流行病学特点及病理类型与免疫荧光之间的关系

目的探讨江苏地区508例肾活检资料的流行病学特点及病理类型与免疫荧光之间的关系。方法回顾性分析508例因肾脏疾病而行肾活检患者的临床资料。结果 508例患者年龄(36.1±13.6)岁;男242例,女266例。狼疮性肾炎(LN)、肾小管-间质疾病(TID)、膜增生性肾小球肾炎(MPGN)、新月体性肾炎(CREGN)、轻微病变(MGA)及IgA肾病(IgAN)女性居多。原发性肾小球肾炎392例,继发性肾小球肾炎109例。最常见病理类型为IgAN、系膜增生性病变(MsPL)、膜性肾病(MN)、MGA、微小病变(MCNS)、局灶阶段性肾小球硬化症(FSGS)、LN。最常见的临床表现为肾病综合征(NS)、肾炎综合征(CNS)。NS中最常见的病理类型为MCNS、MN、MsPL、IgAN、FSGS、MGA;CNS最常见的病理类型为:IgAN、MsPL。IgAN肾组织以IgA沉积为主;DN肾组织83.3%有不同程度及类型的免疫球蛋白沉积;LN肾组织92.3%病例呈"满堂亮"表现。结论江苏地区肾脏疾病男女比例相当,青壮年是高发人群;最常见的肾小球疾病是IgAN;IgAN肾脏组织以IgA沉积为主;最常见的临床表现是NS;江苏北部Alport综合征发病率较其他地区高。     

不同病理类型的肾穿刺活检患者临床分析

目的探讨超声引导下肾组织活检在肾脏疾病诊治中的价值。方法对78例具有肾脏穿刺适应证的肾病患者行超声引导下肾组织活检,并作病理学检查,结合临床特点进行分析。结果78例患者中原发性肾小球肾炎(PGN)55例,占70.5%,病理类型以IgA肾病(IgAN)居多(29.1%),临床表现以肾病综合征(NS)多见(43.6%);继发性肾小球肾炎(SGN)中以狼疮性肾炎(LN)多见(46.7%)。结论超声引导下肾活检对肾脏疾病的诊断、治疗及研究具有非常重要的作用。     

终末期肾病血液透析患者动静脉内瘘失功的危险因素分析及护理对策

目的探讨终末期肾病血液透析患者动静脉内瘘失功的危险因素,以危险因素为导向,总结其发生原因,从根本上加以防护。方法选取2015年2月至2017年2月在我院进行血液透析治疗的终末期肾病患者80例为研究对象,以动静脉内瘘是否失功分为通畅组67例和失功组13例,采用多因素logistic回归分析终末期肾病血液透析动静脉内瘘失功的相关因素。结果单因素分析显示,年龄、血流量、原发病、压迫时间、低血压、感染、甘油三酯、血磷是终末期肾病血液透析动静脉内瘘失功的相关因素(P<0.05)。多因素logistic回归分析显示,血流量<200 ml/min、糖尿病肾病、压迫时间≥30 min、低血压、感染、甘油三酯>1.71 mmol/L是终末期肾病患者血液透析动静脉瘘失功的独立危险因素(P<0.05)。结论影响终末期肾病患者血液透析动静脉内瘘失功的因素较多,应根据这些因素采取护理干预措施,以避免动静脉内瘘失功发生。     

University of Miami Division of Clinical Pharmacology Therapeutic Rounds: ischemic renal disease

Ischemic renal disease (IRD) is defined as a significant reduction in glomerular filtration rate and/or loss of renal parenchyma caused by hemodynamically significant renal artery stenosis. IRD is a common and often overlooked clinical entity that presents in the setting of extrarenal arteriosclerotic vascular disease in older individuals with azotemia. IRD is an important cause of chronic renal failure and end-stage renal disease (ESRD), and many patients with a presumed diagnosis of hypertensive nephrosclerosis may actually have undiagnosed ischemic nephropathy as the cause of their ESRD. The primary reason for establishing the diagnosis of IRD is the hope that correction of a renal artery stenosis will lead to improvement of renal function or a delay in progression to ESRD. There are six typical clinical settings in which the clinician could suspect IRD: acute renal failure caused by the treatment of hypertension, especially with angiotensin-converting enzyme inhibitors; progressive azotemia in a patient with known renovascular hypertension; acute pulmonary edema superimposed on poorly controlled hypertension and renal failure; progressive azotemia in an elderly patient with refractory or severe hypertension; progressive azotemia in an elderly patient with evidence of atherosclerotic disease; and unexplained progressive azotemia in an elderly patient. It is important for the clinician to identify IRD, because IRD represents a potentially reversible cause of chronic renal failure in a hypertensive patient.     

The familial clustering of renal disease and related phenotypes

This article reviews the familial aggregation of chronic kidney diseases including end-stage renal disease and albuminuria, along with variation in glomerular filtration rate. In addition to environmental influences on the progression of nephropathy, epidemiologic evidence in support of the existence of renal failure susceptibility genes is presented.     

Can progression of renal disease be prevented?

Renal disease from a variety of causes often progresses to end-stage renal failure. The progression may be caused by factors accompanying, but not initiating, renal injury. These factors include glomerular hyperfiltration, glomerular hypertension, systemic hypertension, and hyperlipidemia. Studies, primarily in animals, indicate that causative factors may be altered by control of systemic hypertension, dietary protein restriction, administration of angiotensin-converting enzyme inhibitors or calcium channel blockers, and plasma lipid control. Whether such interventions will significantly alter progressive renal disease in humans is, as yet, uncertain.     

Association between urinary VEGFA and renal pathology of IgA nephropathy patients

BackgroundRenal biopsy remains the golden standard for diagnosing and monitoring IgA nephropathy (IgAN). Vascular endothelial growth factor A (VEGFA) was crucial for the survival of glomerular cells. Our aim was to screen the expression pattern of urinary, circulating and renal VEGFA in IgAN patients to reveal their relationship with renal pathology and outcomes.MethodsBaseline VEGFA levels were determined with ELISA, real‐time PCR and immunohistochemistry. Associations between VEGFA expression and clinical–pathological parameters, and renal outcomes were evaluated.ResultsCompared with healthy controls, urinary VEGFA level was obviously elevated in IgAN patients (76.19 ± 63.67 pg/mg Cr vs 146.67 ± 232.71 pg/mg Cr, p = 0.0291) and not correlated with serum VEGFA level. Baseline urinary VEGFA was significantly associated with gender and tubular atrophy/interstitial fibrosis by stepwise multivariate regression analysis. Urinary VEGFA was higher in male patients accompanied with higher serum creatinine, larger proportion of hypertension and recurrent hematuria than in female patients. In the kidney of IgAN patients, VEGFA were robustly expressed in the parietal epithelial cells, podocytes, mesangial cells and tubular epithelial cells. After a follow‐up duration of 38.53 ± 27.14 months, IgAN patients with higher urinary VEGFA level were found to have a poorer renal outcome of renal replacement therapy (HR = 1.027, p = 0.037) or composite outcome (HR = 1.023, p = 0.039) after adjusting for confounders.ConclusionsIncreased urinary VEGFA might reflect certain renal pathology and, although not fully specific, still could be served as a valuable noninvasive indicator in predicting renal progression of IgAN.     

肝肾联合移植的适应证及时机

背景：肝肾联合移植以来,肾功能不全甚至肾功能衰竭已不再是肝脏移植的禁忌症。目的：探寻肝肾联合移植适应证及移植时机,以利合理应用稀缺的实体器官供体。方法：收集接受肝肾联合移植患者15例,回顾性分析其移植前状态与移植后移植肾及原肾恢复情况间的状态。结果与结论：入组15例肝肾联合移植患者均手术顺利,至今存活,随访1.5-8（3.6±1.2）年。入组患者中出现移植肾功能延迟恢复1例,行床旁连续性肾脏替代治疗治疗2周后肾功能逐渐恢复;1例移植前行连续性肾脏替代治疗治疗4周的肝肾综合征患者,移植后2个月行肾图检查提示原肾功能恢复正常;另2例移植前连续性肾脏替代治疗超过6周的肝肾综合征患者,移植后行肾图提示原肾功能未恢复;伴有原发肾病的终末期肝病患者移植前24h尿蛋白〉500mg、肾小球滤过率〈30mL/min或经穿刺活检证实肾小球硬化率〉30%,肝肾联合移植后行肾图提示原肾逐渐失功。移植前行连续性肾脏替代治疗治疗超过6周的肝肾综合征患者,需施行肝肾联合移植;移植前伴有原发肾病的终末期肝病患者,如果24h尿蛋白〉500mg、肾小球滤过率〈30mL/min或经活检证实肾小球硬化率〉30%,需施行肝肾联合移植。     

The renin-angiotensin system and progression of renal disease: from hemodynamics to cell biology

The renal community is faced with an ever increasing number of patients reaching end-stage renal failure. Clinical studies have provided clear evidence that angiotensin-converting enzyme (ACE) inhibitors, and probably also AT1 receptor antagonists, at least in patients suffering from type 2 diabetes, slow disease progression to end-stage renal failure. This protective effect of drugs interfering with the renin-angiotensin system (RAS) are in part independent of reduction in systemic blood pressure, but involve normalization of glomerular hyperperfusion and hyperfiltration, restoration of altered glomerular barrier function, and reduction of stimulated tubular fluid reabsorption. Angiotensin II (ANG II) has emerged in the last decade as a multifunctional cytokine exhibiting many non-hemodynamic properties such as acting as a growth factor and profibrogenic cytokine, and even having proinflammatory properties. This review tries to bridge the classical hemodynamic actions of ANG II in the kidney with the more recently characterized effects of this vasopeptide. Finally, clinical implications are suggested based on data from clinical studies. A thorough understanding of the RAS is important to recognize the potential of nephroprotective strategies through inhibition of its components.     

Antihypertensive therapy and mobilization of renal functional reserve.

The normal kidney can increase its rate of glomerular filtration in response to an acute protein load. It has been suggested that this acute hyperfiltration represents a renal functional reserve (RFR). The RFR has also been proposed to reflect the chronic hyperfiltration found in diabetic patients and animal models of chronic renal failure. The physiologic role of the RFR is still unclear. On the one hand, the availability of an RFR may retard the progression towards end-stage renal failure. On the other hand, sustained hyperfiltration has been implicated as a potential deleterious factor in the progression of renal disease. Antihypertensive drugs used in the management of hypertensive patients with chronic renal disease modify both the systemic and the renal hemodynamics. Depending on their hemodynamic effects, they may thereby alter the ability to mobilize RFR. Today, it is still not clear whether an ideal compound should increase, decrease, or not affect RFR to preserve long-term renal function. Evaluation of the effects of various antihypertensive agents on RFR could become an important aspect of consideration in order to optimize both the control of blood pressure and the capacity of the therapy to prevent deterioration of renal function.